Abstract
Waldenstrom macroglobulinemia (WM) is a B-cell lymphoplasmacytic lymphoma characterized by monoclonal immunoglobulin M protein in the serum and infiltration of bone marrow with lymphoplasmacytic cells. Asymptomatic patients can be observed without therapy. First-line therapy should consist of the monoclonal anti-CD20 antibody, rituximab, given typically in combination with other agents. We prefer dexamethasone, rituximab, cyclophosphamide (DRC) as initial therapy for most patients with symptomatic WM. Other reasonable options are bortezomib, rituximab, dexamethasone (BoRD) or bendamustine plus rituximab (BR). All of these regimens are associated with excellent response and tolerability. Initial therapy is usually administered for 6 months, followed by observation. Response to therapy is assessed using the standard response criteria developed by the International Working Group on Waldenstrom macroglobulinemia. Relapse is almost inevitable in WM but may occur years after initial therapy. In symptomatic patients relapsing more than 1–2 years after initial therapy, the original treatment can be repeated. For relapse occurring sooner, an alternative regimen is used. In select patients, high-dose chemotherapy followed by autologous hematopoietic cell transplantation may be an option at relapse. Options for therapy of relapsed WM besides regimens used in the front-line setting include ibrutinib, purine nucleoside analogs (cladribine, fludarabine), carfilzomib and immunomodulatory agents (thalidomide, lenalidomide).
Highlights
Waldenstrom macroglobulinemia (WM) is defined as a B-cell lymphoplasmacytic lymphoma, characterized by monoclonal immunoglobulin M protein in the serum and infiltration of bone marrow with lymphoplasmacytic cells.[1]
A majority of patients with WM have a recurrent mutation of the MYD88 gene (MYD88 L265P).[2,3]
As all WM cells express CD-20, it is generally agreed upon that first-line therapy should consist of monoclonal antiCD20 antibody, rituximab, alone or preferably in combination
Summary
Waldenstrom macroglobulinemia (WM) is defined as a B-cell lymphoplasmacytic lymphoma, characterized by monoclonal immunoglobulin M protein in the serum and infiltration of bone marrow with lymphoplasmacytic cells.[1]. Several staging systems have been proposed to risk stratify patients with WM and to aid in prognosis (Table 1).[10,14,15] Dhodapkar and colleagues[14] developed a three-parameter staging system for WM based on the results of a multicenter clinical trial conducted by the Southwest Oncology Group. This model uses hemoglobin concentration, β2-microglobulin levels and serum immunoglobulin (Ig) M level to classify patients into four prognostic groups with significantly different 5-year survival rates. Care should be given to avoid treatments that may compromise stem-cell collection in patients who are candidates for autologous hematopoietic cell transplantation (HCT)
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